Safety of Major Abdominal Surgical Procedures in Patients with Hyperimmunoglobulinemia E (Job’s Syndrome): a Changing Paradigm?
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- 作者：Russell C. Langan (1) (2)
Richard M. Sherry (1)
Itzhak Avital (3)
Theo Heller (4)
Carolyn Henderson (5)
Steven M. Holland (5)
Alexandra F. Freeman (5)
Udo Rudloff (1) - 关键词：Job’s syndrome ; Hyperimmunoglobulinemia E ; Immunodeficiency ; Liver resection ; Gastrectomy ; Colon resection
- 刊名：Journal of Gastrointestinal Surgery
- 出版年：2013
- 出版时间：May 2013
- 年：2013
- 卷：17
- 期：5
- 页码：1009-1014
- 全文大小：290KB
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Richard M. Sherry (1)
Itzhak Avital (3)
Theo Heller (4)
Carolyn Henderson (5)
Steven M. Holland (5)
Alexandra F. Freeman (5)
Udo Rudloff (1)
1. Surgery Branch, National Cancer Institute, National Institutes of Health, Bethesda, MD, USA
2. Department of Surgery, Georgetown University Hospital, 3800 Reservoir Road, NW 2 GOR, Washington, DC, 20007, USA
3. Bon Secours Cancer Institute, Richmond, VA, USA
4. National Institute of Diabetes and Digestive and Kidney Diseases, Bethesda, MD, USA
5. National Institute of Allergy and Infectious Disease, National Institutes of Health, CCR, Room 11?N234, 10 Center Drive, Bethesda, MD, 20892-0001, USA
文摘
Introduction Autosomal dominant hyperimmunoglobulinemia E syndrome (HIES), also called Job’s syndrome, is a primary immunodeficiency characterized by the triad of elevated immunoglobulin E levels, eczema, and infections. Its clinical course manifests as recurrent skin and pulmonary infections, and variable skeletal, connective tissue, and vascular abnormalities. There is evidence of abnormal tissue remodeling with pneumatocoeles frequently complicating pyogenic pneumonias and leading to secondary infections that cause the majority of morbidity and mortality. Complications are known to occur after lung surgery with a high frequency of bronchopleural fistulae, but little has been reported concerning abdominal surgeries. Discussion Here, we report on the outcome and safety of two separate complex cases (hepatectomy and subtotal gastrectomy) and document our entire experience with abdominal surgical procedures performed on patients with HIES. Despite initial complications, all patients eventually made a full recovery. Conclusion As HIES patients now frequently live beyond the third and fourth decade, surgical issues similar to those in the general population may increase. Complex surgical procedures can be performed safely and benefit select patients with HIES, but benefit strongly from multidisciplinary teams and awareness of complications related to abnormal healing. We discuss current treatment and potential complications post-operatively in patients with HIES.